Peroxisomes Are Required for Lipid Metabolism and Muscle Function in Drosophila melanogaster

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dc.citation.firstpagee100213en_US
dc.citation.issueNumber6en_US
dc.citation.journalTitlePLoS ONEen_US
dc.citation.volumeNumber9en_US
dc.contributor.authorFaust, Joseph E.en_US
dc.contributor.authorManisundaram, Arvinden_US
dc.contributor.authorIvanova, Pavlina T.en_US
dc.contributor.authorMilne, Stephen B.en_US
dc.contributor.authorSummerville, James B.en_US
dc.contributor.authorBrown, H.Alexen_US
dc.contributor.authorWangler, Michael F.en_US
dc.contributor.authorStern, Michaelen_US
dc.contributor.authorMcNew, James A.en_US
dc.date.accessioned2015-07-09T20:14:49Zen_US
dc.date.available2015-07-09T20:14:49Zen_US
dc.date.issued2014en_US
dc.description.abstractPeroxisomes are ubiquitous organelles that perform lipid and reactive oxygen species metabolism. Defects in peroxisome biogenesis cause peroxisome biogenesis disorders (PBDs). The most severe PBD, Zellweger syndrome, is characterized in part by neuronal dysfunction, craniofacial malformations, and low muscle tone (hypotonia). These devastating diseases lack effective therapies and the development of animal models may reveal new drug targets. We have generated Drosophila mutants with impaired peroxisome biogenesis by disrupting the early peroxin gene pex3, which participates in budding of pre-peroxisomes from the ER and peroxisomal membrane protein localization. pex3 deletion mutants lack detectible peroxisomes and die before or during pupariation. At earlier stages of development, larvae lacking Pex3 display reduced size and impaired lipid metabolism. Selective loss of peroxisomes in muscles impairs muscle function and results in flightless animals. Although, hypotonia in PBD patients is thought to be a secondary effect of neuronal dysfunction, our results suggest that peroxisome loss directly affects muscle physiology, possibly by disrupting energy metabolism. Understanding the role of peroxisomes in Drosophila physiology, specifically in muscle cells may reveal novel aspects of PBD etiology.en_US
dc.identifier.citationFaust, Joseph E., Manisundaram, Arvind, Ivanova, Pavlina T., et al.. "Peroxisomes Are Required for Lipid Metabolism and Muscle Function in Drosophila melanogaster." <i>PLoS ONE,</i> 9, no. 6 (2014) Public Library of Science: e100213. http://dx.doi.org/10.1371/journal.pone.0100213.en_US
dc.identifier.doihttp://dx.doi.org/10.1371/journal.pone.0100213en_US
dc.identifier.urihttps://hdl.handle.net/1911/80864en_US
dc.language.isoengen_US
dc.publisherPublic Library of Scienceen_US
dc.rightsThis is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.en_US
dc.rights.urihttps://creativecommons.org/licenses/by/4.0/en_US
dc.titlePeroxisomes Are Required for Lipid Metabolism and Muscle Function in Drosophila melanogasteren_US
dc.typeJournal articleen_US
dc.type.dcmiTexten_US
dc.type.publicationpublisher versionen_US
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